title: Atypical hyaline vascular-type castleman's disease with thrombocytopenia, anasarca, fever, and systemic lymphadenopathy.
abstract: Recently, atypical Castleman's disease (CD) was reported in Japan. This disease is considered as TAFRO syndrome or non-idiopathic plasmacytic lymphadenopathy (IPL), a constellation of clinical symptoms, namely, thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly without hyper-γ-globulinemia. Histopathologically, this disease is similar to hyaline vascular (HV)-type CD. Here, we present a 43-year-old Japanese woman meeting the clinical criteria of TAFRO syndrome who was successfully treated with combined corticosteroid therapy. She showed a rapidly progressive course of thrombocytopenia, systemic lymphadenopathy, fever, anasarca, and increase in acute inflammatory proteins without hyper-γ-globulinemia. Lymph node biopsy was performed and revealed HV-type CD without human herpes virus 8 infection, which was clinicopathologically compatible with non-IPL. The association of these atypical features with well-known multicentric Castleman's disease (MCD), namely, HV-type histology with systemic lymphadenopathy, marked thrombocytopenia even with a high level of interleukin-6, and increased acute inflammatory proteins without hyper-γ-globulinemia, suggests that TAFRO syndrome as presented in our case is a novel entity, which may have been diagnosed as MCD in the past. To define this novel entity more clearly and to demonstrate its etiology, further nationwide surveys of this syndrome and MCD are needed.
authors.affiliation: Departments of Pathology Head and Neck Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Shikata-cho, Okayama, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Iwaki
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Sato
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.forename: Eisei
authors.initials: E
authors.lastname: Kondo
authors.forename: Kyotaro
authors.initials: K
authors.lastname: Ohno
authors.forename: Mai
authors.initials: M
authors.lastname: Takeuchi
authors.forename: Yorihisa
authors.initials: Y
authors.lastname: Orita
authors.forename: Shinji
authors.initials: S
authors.lastname: Nakao
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yoshino
mesh_terms: D000328:Adult; D005871:Castleman Disease / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D004487:Edema / Q000473:pathology; D005260:Female; D005334:Fever / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008206:Lymphatic Diseases / Q000473:pathology; D013921:Thrombocytopenia / Q000473:pathology